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Orthotopic liver transplantation for acute liver failure secondary to autoimmune hepatitis in a child with autoimmune polyglandular syndrome type 1
Author(s) -
Smith Dominic,
Stringer Mark D.,
Wyatt Judy,
O'Meara Moira,
Davison Suzanne,
Cheetham Tim D.,
McClean Patricia
Publication year - 2002
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1034/j.1399-3046.2002.01063.x
Subject(s) - medicine , autoimmune hepatitis , fulminant hepatic failure , liver transplantation , chronic mucocutaneous candidiasis , fulminant , asymptomatic , autoimmune disease , hypoparathyroidism , liver disease , hepatitis , immunology , transplantation , disease
Autoimmune polyglandular syndrome type 1 (APS‐1) is an autosomal‐recessive condition characterized by hypoparathyroidism, autoimmune Addison's disease, and chronic mucocutaneous candidiasis. Autoimmune hepatitis develops in 10–20% of affected patients and has a variable course ranging from asymptomatic chronic liver disease to lethal fulminant hepatic failure. Liver transplantation has been documented previously in only two patients. We report a 14‐yr‐old boy with APS‐1 who developed acute liver failure secondary to associated autoimmune hepatitis. He did not respond to corticosteroid therapy and was successfully treated with an orthotopic liver transplant.